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Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic

Abstract

Auditory neuropathy spectrum disorder (ANSD) is a particular kind of hearing disorder characterised by normal outer hair cell function and abnormal or absent auditory brain stem responses. Little data are available regarding the prevalence of this condition in healthy newborns. We performed a retrospective medical records review of 791 referrals from universal neonatal hearing screening (UNHS) at a well-baby clinic to investigate the prevalence of ANSD. Hearing screening was performed by automated auditory brain stem response (ABR) testing. A diagnosis of ANSD was established when ABR tracings were absent in the presence of otoacoustic emissions and/or a cochlear microphonic. Amongst 201 infants with confirmed congenital hearing loss, 13 infants were diagnosed with ANSD. The condition was unilateral in six and bilateral in seven infants. A risk factor for hearing loss could be identified in three infants. Abnormalities on magnetic resonance imaging were found in six infants; five of them had cochlear nerve deficiency.

Conclusion: The prevalence of ANSD was 6.5 % amongst well babies with confirmed congenital hearing loss identified through UNHS. The estimated incidence of ANSD in our population of newborns at the well-baby clinic was 0.09/1000 live births. Magnetic resonance revealed an underlying anatomical abnormality in about half of the patients.

What is known:
• Auditory neuropathy dyssynchrony spectrum disorder (ANSD) is a particular form of hearing loss, mostly encountered in neonatal intensive care unit (NICU) graduates.
• Little data are available on the prevalence and risk factors for ANSD in healthy newborns.
What is new:
• The estimated prevalence of ANSD in healthy newborns is 0.09/1000 live births.
• In about half of the healthy newborns with ANSD, a structural abnormality was detected on magnetic resonance imaging of the posterior fossa/brain.

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Abbreviations

AABR:

Automatic ABR

ABR:

Auditory brain stem responses

ASSR:

Auditory steady state responses

CM:

Cochlear microphonic

CMV:

Cytomegalovirus

CND:

Cochlear nerve deficiency

ENT:

Ear-nose-throat

HL:

Hearing loss

MRI:

Magnetic resonance imaging

nHL:

Normal hearing level

NICU:

Neonatal intensive care unit

TOAEs:

Transient evoked otoacoustic emissions

UNHS:

Universal neonatal hearing screening

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Acknowledgments

The authors would like to thank Luc Stappaerts from Kind and Gezin who provided the data on the UNHS screening for Flanders.

Author’s contribution

All authors contributed to data collection, interpretation of the data and preparation of the manuscript.

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Authors

Corresponding author

Correspondence to A. Boudewyns.

Ethics declarations

Funding

None of the authors received any financial support or funding for the present study.

Conflict of interest

An Boudewyns declares that she has no conflict of interest.

Frank Declau declares that he has no conflict of interest.

Jenneke van den Ende declares that she has no conflict of interest.

Anouk Hofkens declares that she has no conflict of interest.

Sara Dirckx declares that she has no conflict of interest.

Paul van de Heyning declares that he has no conflict of interest.

Ethical approval

The study was performed in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from the caregivers of all individual infants included in the study.

Additional information

Communicated by Peter de Winter

Appendix: Cochlear microphonic

Appendix: Cochlear microphonic

The cochlear microphonic is a preneural response from the cochlear outer hair cells. The presence of a cochlear microphonic at or below a sound level that does not evoke a recordable ABR is an indication for ANSD. The cochlear microphonic is a more robust criterion for the diagnosis of ANSD compared to transient evoked otoacoustic emissions because these may disappear with time or may be absent in cases where there is also a conductive component to the hearing loss (such as with middle ear effusion).

The recommended method to detect a CM is the use of separate, replicated runs of condensation and rarefaction polarity at a stimulus level of 80 dB nHL.

A CM is considered present when a waveform appears in the first few milliseconds of the tracing and is the only part of the waveform that reverses polarity from rarefaction to condensation. A control trial is then performed with the sound tube of the insert phone clamped to rule out transducer artefacts.

An example of a cochlear microphonic is provided below Fig. 3. More detailed information may be found at Lightfoot G (ed). 2011. Guidelines for cochlear microphonic testing. NHSP Clinical Group (http://www.thebsa.org.uk/wp-content/uploads/2015/02/CM_Guidance_v2_2109111.pdf).

Fig. 3
figure3

An example of an ABR tracing for the right ear, illustrating a cochlear microphonic. Using alternating click stimuli at intensities of 80 and 70 dB nHL, no ABR tracing is present (red line). However, using runs of condensation and rarefaction polarity at the same stimulus intensities reveals the presence of a cochlear microphonic (black arrow) appearing after 1 ms

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Boudewyns, A., Declau, F., van den Ende, J. et al. Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic. Eur J Pediatr 175, 993–1000 (2016). https://doi.org/10.1007/s00431-016-2735-5

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Keywords

  • Auditory neuropathy spectrum disorder
  • Neonatal hearing screening
  • Hearing loss
  • Auditory brain stem response